Garvin A J, Self S, Sahovic E A, Stuart R K, Marchalonis J J
Department of Pathology and Laboratory Medicine, Medical University of South Carolina, Charleston 29425.
Am J Surg Pathol. 1988 Jan;12(1):64-70. doi: 10.1097/00000478-198801000-00008.
A 32-year-old man received a cadavaric renal transplant in 1975 for end-stage renal disease and, thereafter, was treated with azathioprine and methylprednisolone for chronic immunosuppression. In 1985, he presented with fever and pancytopenia that persisted despite withdrawal of the immunosuppressive agents. Lymph node and liver biopsies demonstrated malignant lymphoma within the sinuses of the node and the sinusoids of the liver. A splenectomy was performed for persistent pancytopenia, and the spleen demonstrated malignant lymphoma of the diffuse mixed large and small cell type exclusively within the cords of the red pulp. The immunophenotype of the tumor cells was obtained by frozen section immunoperoxidase staining with monoclonal antibodies and flow cytometric analysis. The tumor cells were positive for the Pan T cell markers CD3 and CD2, but were negative for the subset markers CD4 and CD8. A DNA hybridization study conducted on the splenic tissue conclusively identified the clonal nature of the malignant T cells by demonstrating rearrangement of the T cell receptor beta gene. In spite of multiple chemotherapeutic regimens, the patient developed increasing peripheral blood involvement and died with disseminated lymphoma. This case appears to be unique in that it is the first report of a chronically immunosuppressed transplant recipient to develop a malignant lymphoma of the mature T cell type, and several of the pathologic features of the tumor have not been observed previously.
一名32岁男性于1975年因终末期肾病接受了尸体肾移植，此后一直使用硫唑嘌呤和甲泼尼龙进行慢性免疫抑制治疗。1985年，他出现发热和全血细胞减少，尽管停用了免疫抑制剂，但症状仍持续存在。淋巴结和肝脏活检显示，在淋巴结窦和肝血窦内存在恶性淋巴瘤。因持续的全血细胞减少进行了脾切除术，脾脏显示弥漫性混合大细胞和小细胞型恶性淋巴瘤仅存在于红髓索内。通过单克隆抗体冷冻切片免疫过氧化物酶染色和流式细胞术分析获得肿瘤细胞的免疫表型。肿瘤细胞对泛T细胞标志物CD3和CD2呈阳性，但对亚群标志物CD4和CD8呈阴性。对脾脏组织进行的DNA杂交研究通过显示T细胞受体β基因重排，最终确定了恶性T细胞的克隆性质。尽管采用了多种化疗方案，患者外周血受累情况仍不断加重，最终死于播散性淋巴瘤。该病例似乎具有独特性，因为这是首例关于长期免疫抑制的移植受者发生成熟T细胞型恶性淋巴瘤的报道，且该肿瘤的一些病理特征此前尚未被观察到。
